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humans (160) 160
frasier-syndrome (127) 127
female (102) 102
male (84) 84
frasier syndrome (80) 80
wt1 proteins - genetics (71) 71
mutation (70) 70
index medicus (68) 68
urology & nephrology (57) 57
wt1 (55) 55
denys-drash syndrome (54) 54
mutations (54) 54
pediatrics (53) 53
child (49) 49
animals (48) 48
wilms-tumor (48) 48
wt1 gene (45) 45
child, preschool (43) 43
adolescent (41) 41
genes, wilms tumor (41) 41
frasier syndrome - genetics (36) 36
nephrotic syndrome (35) 35
expression (33) 33
nephrotic syndrome - genetics (33) 33
infant (32) 32
phenotype (32) 32
denys-drash syndrome - genetics (31) 31
mice (31) 31
focal segmental glomerulosclerosis (29) 29
gene (28) 28
genetic aspects (24) 24
base sequence (23) 23
cell biology (23) 23
glomerulosclerosis, focal segmental - genetics (23) 23
denys-drash-syndrome (22) 22
dna-binding proteins - genetics (22) 22
transcription factors - genetics (22) 22
genetics & heredity (21) 21
gonadoblastoma (21) 21
medicine & public health (21) 21
wilms tumor - genetics (21) 21
alternative splicing (20) 20
endocrinology & metabolism (20) 20
urogenital development (20) 20
wt1 proteins (20) 20
adult (19) 19
wt1 proteins - metabolism (19) 19
biochemistry & molecular biology (18) 18
diffuse mesangial sclerosis (18) 18
infant, newborn (18) 18
wilms' tumor (18) 18
dna mutational analysis (17) 17
kidney transplantation (17) 17
molecular sequence data (17) 17
pseudohermaphroditism (17) 17
wilms-tumor gene (16) 16
kidney failure, chronic - genetics (15) 15
kidney neoplasms - genetics (15) 15
nephropathy (15) 15
disorders of sex development - genetics (14) 14
isoforms (14) 14
kidney development (14) 14
kts splice isoforms (14) 14
urologic and male genital diseases (14) 14
amino acid sequence (13) 13
children (13) 13
exons (13) 13
gene mutations (13) 13
genetic predisposition to disease (13) 13
gonadoblastoma - genetics (13) 13
oncology (13) 13
prevalence (13) 13
splice-site mutations (13) 13
steroid-resistant (13) 13
suppressor gene (13) 13
syndrome (13) 13
tumors (13) 13
analysis (12) 12
frasier syndrome - complications (12) 12
gene expression regulation, developmental (12) 12
research (12) 12
sex determination (12) 12
transcription factor (12) 12
urogenital system (12) 12
drash syndrome (11) 11
exons - genetics (11) 11
gonadal dysgenesis (11) 11
gonadal-dysgenesis (11) 11
karyotyping (11) 11
kidney diseases - genetics (11) 11
nephrin (11) 11
nephrotic syndrome - drug therapy (11) 11
subnuclear localization (11) 11
cell line (10) 10
developmental biology (10) 10
disease progression (10) 10
female genital diseases and pregnancy complications (10) 10
frasier syndrome - diagnosis (10) 10
gene expression (10) 10
genes (10) 10
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1. Full Text Genotype–phenotype associations in WT1 glomerulopathy
by Lipska, Beata S and Ranchin, Bruno and Iatropoulos, Paraskevas and Gellermann, Jutta and Melk, Anette and Ozaltin, Fatih and Caridi, Gianluca and Seeman, Tomas and Tory, Kalman and Jankauskiene, Augustina and Zurowska, Aleksandra and Szczepanska, Maria and Wasilewska, Anna and Harambat, Jerome and Trautmann, Agnes and Peco-Antic, Amira and Borzecka, Halina and Moczulska, Anna and Saeed, Bassam and Bogdanovic, Radovan and Kalyoncu, Mukaddes and Simkova, Eva and Erdogan, Ozlem and Vrljicak, Kristina and Teixeira, Ana and Azocar, Marta and Schaefer, Franz and PodoNet Consortium
Kidney International, ISSN 0085-2538, 05/2014, Volume 85, Issue 5, pp. 1169 - 1178
mutations cause a wide spectrum of renal and extrarenal manifestations. Here we evaluated disease prevalence, phenotype spectrum, and genotype–phenotype... 
Denys–Drash syndrome | diffuse mesangial sclerosis | Frasier syndrome | steroid-resistant nephrotic syndrome | focal segmental glomerulosclerosis | WT1 | Denys-Drash syndrome | Steroid-resistant nephrotic syndrome | Diffuse mesangial sclerosis | Focal segmental glomerulosclerosis | STEROID-RESISTANT | SPLICE-SITE MUTATIONS | RESISTANT NEPHROTIC SYNDROME | BINDING ACTIVITY | SUPPRESSOR GENE WT1 | WILMS-TUMOR | PROTEIN WT1 | UROLOGY & NEPHROLOGY | FRASIER-SYNDROME | EXPRESSION | Prevalence | Prognosis | Glomerulosclerosis, Focal Segmental - genetics | Humans | Child, Preschool | Infant | Male | Nephrotic Syndrome - genetics | Genetic Testing - methods | Nephrotic Syndrome - congenital | Incidence | Renal Insufficiency, Chronic - epidemiology | Renal Insufficiency, Chronic - therapy | Time Factors | DNA Mutational Analysis | Renal Insufficiency, Chronic - genetics | Female | Registries | Renal Insufficiency, Chronic - diagnosis | Nephrotic Syndrome - therapy | Child | Nephrotic Syndrome - diagnosis | WT1 Proteins - genetics | Genetic Predisposition to Disease | Genetic Association Studies | Glomerulosclerosis, Focal Segmental - epidemiology | Risk Factors | Disease Progression | Glomerulosclerosis, Focal Segmental - diagnosis | Phenotype | Age of Onset | Nephrotic Syndrome - epidemiology | Glomerulosclerosis, Focal Segmental - therapy | Mutation
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2. Full Text The podocyte as a target: cyclosporin A in the management of the nephrotic syndrome caused by WT1 mutations
by Stefanidis, Constantinos J and Querfeld, Uwe
European Journal of Pediatrics, ISSN 0340-6199, 11/2011, Volume 170, Issue 11, pp. 1377 - 1383
Children with steroid-resistant nephrotic syndrome secondary to WT1-associated glomerulopathies (WT1-GP) were considered unresponsive to cyclosporin A (CsA).... 
Pediatrics | Frasier syndrome | Medicine & Public Health | WT1-associated glomerulopathies | Denys–Drash syndrome | Steroid-resistant nephrotic syndrome | Wilms’ tumour suppressor gene ( WT1 ) mutations | Cyclosporin A | Denys-Drash syndrome | Wilms' tumour suppressor gene (WT1) mutations | APOPTOSIS | SEGMENTAL GLOMERULOSCLEROSIS | STEROID-RESISTANT | INTERLEUKIN-10 | WILMS-TUMOR | SUPPRESSOR PROTEIN WT1 | MESANGIAL SCLEROSIS | PLCE1 | PEDIATRICS | FRASIER-SYNDROME | TRANSCRIPTIONAL REGULATION | Age Factors | Cyclosporine - pharmacology | Humans | Child, Preschool | Infant | Male | Nephrotic Syndrome - genetics | Denys-Drash Syndrome - genetics | Nephrotic Syndrome - drug therapy | Molecular Targeted Therapy | Frasier Syndrome - genetics | Female | Angiotensin-Converting Enzyme Inhibitors - pharmacology | Drug Therapy, Combination | Denys-Drash Syndrome - drug therapy | Immunosuppressive Agents - pharmacology | Drug Resistance | Immunosuppressive Agents - administration & dosage | Infant, Newborn | Angiotensin-Converting Enzyme Inhibitors - administration & dosage | Methylprednisolone - pharmacology | Genes, Wilms Tumor | Podocytes - drug effects | Nephrotic Syndrome - physiopathology | Cyclosporine - administration & dosage | Frasier Syndrome - drug therapy | Mutation | Methylprednisolone - administration & dosage | Enzymes | Enzyme inhibitors | Genes | Nephrotic syndrome | Genetic aspects | Physicians (General practice) | Cyclosporine
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3. Full Text Wilms’ tumour 1 gene mutations in South Indian children with steroid-resistant nephrotic syndrome
by Kumar, Aravind Selvin and Srilakshmi, R and Karthickeyan, S.M.K and Balakrishnan, K and Padmaraj, R and Senguttuvan, Prabha
Indian Journal of Medical Research, ISSN 0971-5916, 2016, Volume 144, Issue August, pp. 276 - 280
Background & objectives: Clinically, nephrotic syndrome (NS) is a diverse group of symptoms; about 20 per cent of NS cases are resistant to steroid treatment,... 
Denys-drash syndrome | Wilms’ tumour 1 | Frasier syndrome | Steroid-resistant nephrotic syndrome | Focal segmental glomerulosclerosis | Indian children | MEDICINE, RESEARCH & EXPERIMENTAL | ASSOCIATIONS | MANAGEMENT | WILMS-TUMOR-1 GENE | Wilms' tumour 1 | PHENOTYPE | PREVALENCE | IMMUNOLOGY | focal segmental glomerulosclerosis | Denys-Drash syndrome | MEDICINE, GENERAL & INTERNAL | WT1 GENE | steroid-resistant nephrotic syndrome | DISEASES | SPLICE-SITE MUTATION | GONADAL-DYSGENESIS | FRASIER-SYNDROME | Glomerulosclerosis, Focal Segmental - genetics | Humans | Kidney Failure, Chronic - drug therapy | Child, Preschool | Gonads - pathology | Nephrotic Syndrome - pathology | Male | Nephrotic Syndrome - genetics | Nephrotic Syndrome - drug therapy | Drug Resistance - genetics | Kidney Failure, Chronic - pathology | Glomerulosclerosis, Focal Segmental - drug therapy | Female | Steroids - therapeutic use | Mutation | Gonads - surgery | Child | WT1 Proteins - genetics | Glomerulosclerosis, Focal Segmental - pathology | Gene mutations | Genetic aspects | Nephrotic syndrome | Children | Nephroblastoma | Health aspects | Diseases | Genotype & phenotype | Nephrology | Biopsy | Boys | Females | Girls | Deoxyribonucleic acid--DNA | Age | Children & youth | Original | Denys-Drash syndrome - focal segmental glomerulosclerosis - Frasier syndrome - Indian children - steroid-resistant nephrotic syndrome - Wilms′ tumour 1
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4. Full Text WT1 and glomerular diseases
by Niaudet, Patrick and Gubler, Marie-Claire
Pediatric Nephrology, ISSN 0931-041X, 11/2006, Volume 21, Issue 11, pp. 1653 - 1660
The WT1 gene encodes a zinc finger transcription factor involved in kidney and gonadal development and, when mutated, in the occurrence of kidney tumor and... 
Denys-Drash syndrome | Frasier syndrome | WT1 gene | Wilms’ tumor | Diffuse mesangial sclerosis | Glomerular disease | FSGS | Wilms' tumor | diffuse mesangial sclerosis | SUPPRESSOR GENE | WILMS-TUMOR GENE | glomerular disease | UROGENITAL DEVELOPMENT | SPLICE-SITE MUTATION | UROLOGY & NEPHROLOGY | PEDIATRICS | FRASIER-SYNDROME | NEPHROTIC SYNDROME | FOCAL SEGMENTAL GLOMERULOSCLEROSIS | TRANSCRIPTION FACTOR | DENYS-DRASH-SYNDROME | Wilms Tumor - genetics | Kidney Diseases - pathology | Humans | Frasier Syndrome - pathology | Male | Nephrotic Syndrome - genetics | Denys-Drash Syndrome - genetics | Genetic Counseling | Kidney Diseases - genetics | Wilms Tumor - pathology | Chromosomes, Human, X | Frasier Syndrome - genetics | Sclerosis - genetics | Chromosomes, Human, Y | Glomerular Mesangium - pathology | Genes, Wilms Tumor | Sclerosis - pathology | Female | Mutation | Child | Denys-Drash Syndrome - pathology
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5. Full Text Genotype/Phenotype Correlation in Nephrotic Syndrome Caused by WT1 Mutations
by Gil Chernin and Virginia Vega-Warner and Dominik S. Schoeb and Saskia F. Heeringa and Bugsu Ovunc and Pawaree Saisawat and Roxana Cleper and Fatih Ozaltin and Friedhelm Hildebrandt and and Members of the GPN Study Group and GPN Study Grp and Members of the GPN Study Group and and Members of the GPN Study Group
Clinical Journal of the American Society of Nephrology, ISSN 1555-9041, 09/2010, Volume 5, Issue 9, pp. 1655 - 1662
Background and objectives: The risk of developing Wilms tumor (WT) can be present or absent in patients with nephrotic syndrome (NS) caused by WT1 mutations.... 
TUMOR SUPPRESSOR GENE | STEROID-RESISTANT | NPHS2 | UROLOGY & NEPHROLOGY | DRASH SYNDROME | PSEUDOHERMAPHRODITISM | FRASIER-SYNDROME | WILMS-TUMOR | DIFFUSE MESANGIAL SCLEROSIS | Kidney Failure, Chronic - mortality | Sequence Deletion | Kidney Neoplasms - genetics | Wilms Tumor - genetics | United States | Humans | Child, Preschool | Infant | Male | Nephrotic Syndrome - genetics | Mutation, Missense | Kidney Failure, Chronic - ethnology | Frasier Syndrome - genetics | Time Factors | DNA Mutational Analysis | Karyotyping | Female | Child | Nephrotic Syndrome - ethnology | Wilms Tumor - ethnology | Kidney Neoplasms - ethnology | Genetic Predisposition to Disease | Genetic Association Studies | Nephrotic Syndrome - mortality | Risk Assessment | Wilms Tumor - mortality | Introns | Risk Factors | Kaplan-Meier Estimate | Gonadoblastoma - mortality | Codon, Nonsense | Frasier Syndrome - mortality | Kidney Neoplasms - mortality | Disease Progression | Frasier Syndrome - ethnology | Gonadoblastoma - ethnology | Kidney Failure, Chronic - genetics | Phenotype | Turkey | Genes, Wilms Tumor | Gonadoblastoma - genetics | Pedigree | Age of Onset | Israel | Mutation | Original
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6. Full Text Malformation syndromes associated with disorders of sex development
by Hutson, John M and Grover, Sonia R and O'Connell, Michele and Pennell, Samuel D
Nature Reviews Endocrinology, ISSN 1759-5029, 2014, Volume 10, Issue 8, pp. 476 - 487
When embryological development of the internal and/or external genitalia is disrupted, the patient presents with a disorder of sex development (DSD) in the... 
46,XY DISORDERS | LEMLI-OPITZ-SYNDROME | TESTICULAR DESCENT | ENDOCRINOLOGY & METABOLISM | CLINICAL CHARACTERISTICS | KLINEFELTER SYNDROME | KALLMANN-SYNDROME | ANTI-MULLERIAN HORMONE | OF-FUNCTION MUTATIONS | TURNER-SYNDROME | CONGENITAL ABSENCE | Genitalia - abnormalities | Gonadal Dysgenesis - genetics | Humans | Hypertelorism - genetics | Hypospadias - genetics | Male | Cleft Palate - genetics | Denys-Drash Syndrome - genetics | Disorders of Sex Development - genetics | Esophagus - abnormalities | Frasier Syndrome - genetics | Hypogonadism - genetics | Mental Retardation, X-Linked - genetics | Animals | Gonadal Dysgenesis, 46,XY - genetics | Turner Syndrome - genetics | Female | Genetic Diseases, X-Linked - genetics | alpha-Thalassemia - genetics | WAGR Syndrome - genetics | Embryonic development | Gene mutations | Genetic aspects | Research | Health aspects | Sexual disorders | Risk factors
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7. Full Text Laparoscopically removed streak gonad revealed gonadoblastoma in frasier syndrome
by Hashimoto, Kazunori and Horibe, Yu and Ezaki, Jiro and Kanno, Toshiyuki and Takahashi, Nobuko and Akizawa, Yoshika and Matsui, Hideo and Yamamoto, Tomoko and Shibata, Noriyuki
Anticancer Research, ISSN 0250-7005, 07/2017, Volume 37, Issue 7, pp. 3975 - 3979
Background: Frasier syndrome (FS) is characterized by gonadal dysgenesis and progressive nephropathy caused by mutation in the Wilm's tumor gene (WT1). We... 
Gonadoblastoma | Case report | Streak gonad | WT1 gene mutation | Fraiser syndrome | ONCOLOGY | DYSGENESIS | case report | DISORDERS | gonadoblastoma | streak gonad | Ovariectomy | Humans | Tomography, X-Ray Computed | Salpingectomy | Frasier Syndrome - genetics | Ovarian Neoplasms - diagnostic imaging | Gonadoblastoma - diagnostic imaging | Frasier Syndrome - surgery | Adolescent | Gonadal Dysgenesis, 46,XY | Female | Mutation | Frasier Syndrome - complications | WT1 Proteins - genetics
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8. Full Text WAGR Syndrome: A Clinical Review of 54 Cases
by Fischbach, Bernard V and Trout, Kelly L and Lewis, Julia and Luis, Catherine A and Sika, Mohammed
Pediatrics, ISSN 0031-4005, 10/2005, Volume 116, Issue 4, pp. 984 - 988
WAGR syndrome is a rare genetic disorder characterized by a de novo deletion of 11p13 and is clinically associated with Wilms' tumor, aniridia, genitourinary... 
Frasier syndrome | WILMS TUMOR ASSOCIATION | DENYS-DRASH | PAX6 | Wilms' tumor | aniridia | PAX6 MUTATION | DELETION | mental retardation | Denys-Drash syndrome | WAGR syndrome | GENE | WT1 | genitourinary abnormalities | FSGS | PEDIATRICS | 11P13 | Child | WAGR Syndrome - diagnosis | Humans | WAGR Syndrome - genetics | Aniridia | Genitourinary abnormalities | Mental retardation | Case studies | Eye | Pediatrics | Abnormalities | Heredity | Medical disorders | Children & youth
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9. Full Text Spectrum of mutations in Chinese children with steroid-resistant nephrotic syndrome
by Wang, Fang and Zhang, Yanqin and Mao, Jianhua and Yu, Zihua and Yi, Zhuwen and Yu, Li and Sun, Jun and Wei, Xiuxiu and Ding, Fangrui and Zhang, Hongwen and Xiao, Huijie and Yao, Yong and Tan, Weizhen and Lovric, Svjetlana and Ding, Jie and Hildebrandt, Friedhelm
Pediatric Nephrology, ISSN 0931-041X, 07/2017, Volume 32, Issue 7, pp. 1181 - 1192
The aim of this study was to elucidate whether genetic screening test results of pediatric patients with steroid-resistant nephrotic syndrome (SRNS) vary with... 
Chinese | Steroid-resistant nephrotic syndrome | Children | Gene | GENE-MUTATIONS | GLOMERULAR PROTEIN | NPHS2 MUTATIONS | UROGENITAL DEVELOPMENT | WT1 | IMMUNOSUPPRESSION | UROLOGY & NEPHROLOGY | PEDIATRICS | PODOCIN | JAPANESE PATIENTS | FRASIER-SYNDROME | FOCAL SEGMENTAL GLOMERULOSCLEROSIS | Glucocorticoids - therapeutic use | Protein Kinases - genetics | Humans | TRPC6 Cation Channel - genetics | Asian Continental Ancestry Group - genetics | Child, Preschool | Infant | Male | Nephrotic Syndrome - genetics | Mutation, Missense | Nephrotic Syndrome - drug therapy | DNA Mutational Analysis - methods | Genetic Testing - methods | Nephrotic Syndrome - congenital | China | Female | Child | Intracellular Signaling Peptides and Proteins - genetics | WT1 Proteins - genetics | Infant, Newborn | Genetic Predisposition to Disease | Membrane Proteins - genetics | Proteinuria - genetics | Drug Resistance - genetics | Homozygote | Adolescent | Age of Onset | Glucocorticoids - pharmacology | High-Throughput Nucleotide Sequencing | Sequence Analysis, DNA - methods | Cohort Studies | Usage | Care and treatment | Gene mutations | Analysis | Nephrotic syndrome | Nucleotide sequencing | Research | Diagnosis | Pediatric research | Genetic screening | DNA sequencing | Pediatrics | Missense mutation | Etiology | Alport syndrome | Mutation | Minority & ethnic groups | Age | Proteinuria
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10. Full Text WT1 mutations in nephrotic syndrome revisited. High prevalence in young girls, associations and renal phenotypes
by Aucella, Filippo and Bisceglia, Luigi and De Bonis, Patrizia and Gigante, Maddalena and Caridi, Gianluca and Barbano, Giancarlo and Mattioli, Gerolamo and Perfumo, Francesco and Gesualdo, Loreto and Ghiggeri, Gian Marco
Pediatric Nephrology, ISSN 0931-041X, 10/2006, Volume 21, Issue 10, pp. 1393 - 1398
WT1 mutations have been considered a rare cause of nephrotic syndrome but recent reports challenge this assumption. Exclusion of inherited forms is a basic... 
Denys-Drash syndrome | Frasier syndrome | Nephrotic syndrome | Focal segmental glomerulosclerosis | WT1 | ISOFORMS | STEROID-RESISTANT | WILMS-TUMOR-1 GENE | EXON-9 | 46,XX FEMALE | focal segmental glomerulosclerosis | nephrotic syndrome | WILMS-TUMOR | DISEASES | SPLICE-SITE MUTATION | UROLOGY & NEPHROLOGY | PEDIATRICS | FRASIER-SYNDROME | Genetic Testing | Prevalence | Age Factors | Kidney - pathology | Humans | Child, Preschool | Male | Nephrotic Syndrome - genetics | Denys-Drash Syndrome - genetics | Nephrotic Syndrome - drug therapy | Frasier Syndrome - genetics | DNA Mutational Analysis | Karyotyping | Adult | Female | Steroids - pharmacology | Child | WT1 Proteins - genetics | Drug Resistance | Frasier Syndrome - pathology | Nephrotic Syndrome - pathology | Exons - genetics | Mutation - genetics | Phenotype | Adolescent | Sex Factors | Denys-Drash Syndrome - pathology
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11. Full Text A familial WT1 mutation associated with incomplete Denys–Drash syndrome
by Zhu, Chunhua and Zhao, Fei and Zhang, Weizhen and Wu, Hongmei and Chen, Ying and Ding, Guixia and Zhang, Aihua and Huang, Songming
European Journal of Pediatrics, ISSN 0340-6199, 10/2013, Volume 172, Issue 10, pp. 1357 - 1362
Denys–Drash syndrome (DDS) is a rare disorder characterized by nephropathy, male pseudohermaphroditism, and wilms tumor. Cases are thought to arise... 
Pediatrics | Frasier syndrome | Medicine & Public Health | Denys–Drash syndrome | Family | WT1 gene mutation | Children | Denys-Drash syndrome | GENOTYPE/PHENOTYPE CORRELATIONS | GLOMERULAR-DISEASES | NEPHROPATHY | EXON-1 | WILMS-TUMOR | GENE | GERMLINE MUTATIONS | PEDIATRICS | PSEUDOHERMAPHRODITISM | NEPHROTIC SYNDROME | EXPRESSION | Genetic Predisposition to Disease | Humans | Child, Preschool | Exons - genetics | Female | Male | Denys-Drash Syndrome - genetics | Mutation, Missense | Disorders of Sex Development - genetics | WT1 Proteins - genetics | Hernia | Genetic aspects | Gene mutations | Genes | Tumors
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12. Full Text Neoplasms and pathology of sexual developmental disorders (intersex)
by Robboy, Stanley J and Jaubert, Francis
Pathology, ISSN 0031-3025, 02/2007, Volume 39, Issue 1, pp. 147 - 163
Continuing new insights into the biology of sexual development and advances in chromosome analysis have led to early identification and prompt treatment of the... 
Leydig cell deficiency | androgen receptor disorders | maternal ingestion of progestins or androgens | Smith-Lemli-Opitz syndrome | defect in müllerian inhibiting system | mixed gonadal dysgenesis | congenital lipoid adrenal hyperplasia | Denys-Drash syndrome | dysgenetic male pseudohermaphroditism | Klinefelter’s syndrome | Adrenogenital syndrome | defect in the Wilms’ tumour suppressor (WT1) gene | Turner’s syndrome | Frasier’s syndrome | disorders of sexual development | XX male and XY female syndrome (sex reversal) | androgen insensitivity syndromes | testicular regression syndrome | pure gonadal dysgenesis | sexual ambiguity | true hermaphroditism | male pseudohermaphroditism | maternal virilising lesions | 5-alpha-reductase type 2 deficiency | gonadoblastoma | placental aromatase defect | Frasier's syndrome | Turner's syndrome | defect in the Wilms' tumour suppressor (WT1) gene | Klinefelter's syndrome | Maternal ingestion of progestins or androgens | Placental aromatase defect | Maternal virilising lesions | Testicular regression syndrome | Male pseudohermaphroditism | defect in mullerian inhibiting system | LEMLI-OPITZ-SYNDROME | CONGENITAL ADRENAL-HYPERPLASIA | Denys -Drash syndrome | PATHOLOGY | Y-CHROMOSOME SEQUENCES | ANDROGEN INSENSITIVITY SYNDROME | LEYDIG-CELL TUMOR | MIXED GONADAL-DYSGENESIS | MULLERIAN DUCT SYNDROME | adrenogenital syndrome | CARCINOMA IN-SITU | TURNER-SYNDROME PATIENTS | FRASIER-SYNDROME | Disorders of Sex Development - pathology | Humans | Disorders of Sex Development - complications | Female | Male | Neoplasms, Germ Cell and Embryonal - complications | Neoplasms, Germ Cell and Embryonal - pathology | Disorders of Sex Development - classification | Gonadal Dysgenesis, pathology | Gonadoblastoma, genetics | Adrenal Hyperplasia, Congenital, physiopathology | Hermaphroditism, pathology | Turner Syndrome, genetics | Genitalia, Male, pathology | Hermaphroditism, genetics | Klinefelter Syndrome, pathology | Sex Differentiation Disorders, physiopathology | Maternal Exposure | Receptors, Androgen | Sertoli Cells | Gonadoblastoma, pathology | 17-Hydroxysteroid Dehydrogenases, deficiency | Placenta, enzymology | Virilism, physiopathology | Ovary, pathology | Adrenogenital Syndrome, physiopathology | Sex Differentiation Disorders, genetics | Karyotyping | Gonadal Dysgenesis, physiopathology | Testicular Neoplasms, pathology | Gonadal Dysgenesis, genetics | Testosterone 5-alpha-Reductase, deficiency | Genitalia, Female, pathology | Aromatase, deficiency | Turner Syndrome, pathology | Sex Differentiation Disorders, pathology | Mullerian Ducts, pathology | Sex Cord-Gonadal Stromal Tumors, pathology | Pregnancy | Dehydroepiandrosterone | Leydig Cells | Phenotype | Testis, pathology | Ovarian Neoplasms, pathology | Testosterone, biosynthesis | WT1 Proteins, genetics | Cholesterol, biosynthesis | Klinefelter Syndrome, genetics | Neoplasms, Gonadal Tissue, pathology
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13. Full Text OC8 High prevalence of wt1 gene mutations in patients with steroid-resistant nephrotic syndrome
by Sladkov, Dmitry and Savostyanov, Kirill and Nikitin, Alexey and Zhurkova, Natalya and Pakhomov, Alexander and Rykunova, Anastasia and Anan’in, Peter and Tsygin, Alexey
Archives of Disease in Childhood, ISSN 0003-9888, 06/2019, Volume 104, Issue Suppl 3, p. A4
Background and aimsSteroid-resistant nephrotic syndrome (SRNS) is the rare disease and holds a special place in structure of nephrotic syndrome in children, it... 
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14. Full Text Gonadal tumor in Frasier syndrome: A review and classification
by Ezaki, Jiro and Hashimoto, Kazunori and Asano, Tatsuo and Kanda, Shoichiro and Akioka, Yuko and Hattori, Motoshi and Yamamoto, Tomoko and Shibata, Noriyuki
Cancer Prevention Research, ISSN 1940-6207, 04/2015, Volume 8, Issue 4, pp. 271 - 276
Frasier syndrome is a rare inherited disease characterized by steroid-resistant nephrotic syndrome, gonadal tumor, and male pseudohermaphroditism (female... 
WT1 GENE | ONCOLOGY | 46,XY PHENOTYPIC MALE | SPLICE-SITE MUTATION | DYSGENESIS | NEPHROTIC SYNDROME | DYSGERMINOMA | FOCAL SEGMENTAL GLOMERULOSCLEROSIS | GIRLS | GONADOBLASTOMA | FEMALE | Humans | Neoplasms, Germ Cell and Embryonal - etiology | Frasier Syndrome - pathology | Gonads - pathology | Neoplasms, Germ Cell and Embryonal - classification | Female | Male | Frasier Syndrome - complications | Neoplasms, Germ Cell and Embryonal - pathology
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